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March 1989

Clinics in Dermatology: Pseudoxanthoma Elasticum

Arch Dermatol. 1989;125(3):446. doi:10.1001/archderm.1989.01670150136028

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Pseudoxanthoma elasticum (PXE) is a puzzling heritable disorder of elastic fibers. Even though the disease has been recognized for more than a hundred years, little is known of its natural history, and essentially no treatment is available. Moreover, this is a disorder that often goes undiagnosed for needlessly long periods, and, when diagnosed, either false or misleading information is often given to the patient, causing unwarranted anxiety. This sharply focused textbook addresses primarily the clinical facets of PXE. Specifically, the author, Dr Neldner, shares the wealth of his clinical expertise on this disease by summarizing data on 100 patientswith PXE who were followed up over the ten-year period from 1973 to 1983. The observations dispel many myths concerning PXE and clarify a variety of prognostic features, among which are the following: the overall survivorship is probably better than has been previously suggested; in most cases, PXE is inherited as an

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